Browsing by Issue Date, starting with "2014-12"
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- Downbeat nystagmus elicited by eyelid closurePublication . Lemos, J; Pereira, D; Amorim, M; Santiago, B; Paiva, A; Cunha, LWe describe a patient with downbeat nystagmus (DBN) evoked only by eye closure. Brain and spinal cord magnetic resonance imaging revealed a T2 paramedian lesion in the left lower basis pontis and other white matter lesions consistent with multiple sclerosis. One potential mechanism for DBN in this case involves transverse ephaptic spread of excitation from areas that subserve coordinated lid closure to the decussating ventral tegmental tract.
- Pretransplant biopsy in expanded criteria donors: do we really need it?Publication . Tavares da Silva, E; Oliveira, R; Castelo, D; Marques, V; Sousa, V; Moreira, P; Simões, P; Bastos, C; Figueiredo, A; Mota, AAbstract INTRODUCTION: Renal transplantation is the best treatment for end-stage renal disease, including when using expanded criteria donors (ECD) kidneys. However, these suboptimal kidneys should be evaluated rigorously to meet their usefulness. Opinions differ about the best way to evaluate them. MATERIALS AND METHODS: We retrospectively reviewed kidneys from ECD harvested by a single academic institution between January 2008 and September 2013. Needle biopsies were performed at the time of the harvest when considered relevant by the transplant team. Two pathologists where responsible for their analysis; the Remuzzi classification has been used in all cases. RESULTS: We evaluated 560 ECD kidneys. Biopsies were made in 197 (35.2%) organs, 20 of which were considered not usable and 36 good only for double transplantation. Sixty-three kidneys (11.3%) were discarded by the transplant team based on the biopsy result and clinical criteria. Donors who underwent a biopsy were older (P < .001) and had a worse glomerular filtration rate (GFR; P = .001). Comparing donors approved and rejected by the biopsy, the rejected donors were heavier (P = .003) and had a lower GFR (P = .002). Cold ischemia time was longer for the biopsy group (P < .001). Regarding graft function, the biopsy overall score correlated with the transplant outcome in the short and long term. Separately, glomeruli and interstitium scores were correlated with recipient's GFR in the earlier periods (3 months; P = .025 and .037), and the arteries and tubules correlated with GFR in the longer term (at 3 years P = .004 and .010). CONCLUSION: The decision on the usability of ECD grafts is complex. At our center, we chose a mixed approach based on donor risk. Low-risk ECD do not require biopsy. In more complex situations, especially older donors or those with a lower GFR, prompted a pretransplant biopsy. The biopsy results proved to be useful as they relate to subsequent transplant outcomes, thereby allowing us to exclude grafts whose function would most probably be less than optimal.
- Aging is Associated with Impaired Renal Function, INF-gamma Induced Inflammation and with Alterations in Iron Regulatory Proteins Gene ExpressionPublication . Costa, E; Fernandes, J; Ribeiro, S; Sereno, J; Garrido, P; Rocha-Pereira, P; Coimbra, S; Catarino, C; Belo, L; Bronze-da-Rocha, E; Vala, H; Alves, R; Reis, F; Santos-Silva, AOur aim was to contribute to a better understanding of the pathophysiology of anemia in elderly, by studying how aging affects renal function, iron metabolism, erythropoiesis and the inflammatory response, using an experimental animal model. The study was performed in male Wistar, a group of young rats with 2 months age and an old one with 18 months age. Old rats presented a significant higher urea, creatinine, interferon (INF)-gamma, ferritin and soluble transferrin receptor serum levels, as well as increased counts of reticulocytes and RDW. In addition, these rats showed significant lower erythropoietin (EPO) and iron serum levels. Concerning gene expression of iron regulatory proteins, old rats presented significantly higher mRNA levels of hepcidin (Hamp), transferrin (TF), transferrin receptor 2 (TfR2) and hemojuvelin (HJV); divalent metal transporter 1 (DMT1) mRNA levels were significantly higher in duodenal tissue; EPO gene expression was significantly higher in liver and lower in kidney, and the expression of the EPOR was significantly higher in both liver and kidney. Our results showed that aging is associated with impaired renal function, which could be in turn related with the inflammatory process and with a decline in EPO renal production. Moreover, we also propose that aging may be associated with INF-gamma-induced inflammation and with alterations upon iron regulatory proteins gene expression.
- Long-term follow-up of asymptomatic or mildly symptomatic patients with severe degenerative mitral regurgitation and preserved left ventricular functionPublication . Coutinho, GF; Garcia, AL; Correia, PM; Branco, C; Antunes, MJOBJECTIVES: The timing for mitral valve surgery in asymptomatic patients with severe mitral regurgitation and preserved left ventricular function remains controversial. We analyzed the immediate and long-term outcomes of these patients after surgery. METHODS: From January 1992 to December 2012, 382 consecutive patients with severe chronic degenerative mitral regurgitation, with no or mild symptoms, and preserved left ventricular function (ejection fraction ≥ 60%) were submitted to surgery and followed for up to 22 years (3209 patient-years). Patients with associated surgeries, other than tricuspid valve repair, were excluded. Cox proportional-hazard survival analysis was performed to determine predictors of late mortality and mitral reoperation. Subgroup analysis involved patients with atrial fibrillation or pulmonary hypertension. RESULTS: Mitral valvuloplasty was performed in 98.2% of cases. Thirty-day mortality was 0.8%. Overall survival at 5, 10, and 20 years was 96.3% ± 1.0%, 89.7% ± 2.0%, and 72.4% ± 5.8%, respectively, and similar to the expected age- and gender-adjusted general population. Patients with atrial fibrillation/pulmonary hypertension had a 2-fold risk of late mortality compared with the remaining patients (hazard ratio, 2.54; 95% confidence interval, 1.17-4.80; P = .018). Benefit was age-dependent only in younger patients (<65 years; P = .016). Patients with atrial fibrillation/pulmonary hypertension (hazard ratio, 4.20, confidence interval, 1.10-11.20; P = .037) and patients with chordal shortening were at increased risk for reoperation, whereas patients with P2 prolapse (hazard ratio, 0.06; confidence interval, 0.008-0.51; P = .037) and patients with myxomatous valves (hazard ratio, 0.072; confidence interval, 0.008-0.624; P = .017) were at decreased risk. CONCLUSIONS: Mitral valve repair can be achieved in the majority of patients with low mortality (<1%) and excellent long-term survival. Patients with atrial fibrillation/pulmonary hypertension had compromised long-term survival, particularly younger patients (aged <65 years), and are at increased risk of mitral reoperation.
- Gender mismatch between donor and recipient is a factor of morbidity but does not condition survival after cardiac transplantationPublication . Correia, P; Prieto, D; Batista, M; Antunes, MJWe intended to evaluate the influence of sex mismatch between donor and recipient, which is still under much debate, on survival and comorbidities after cardiac transplantation. From November 2003 to December 2013, a total of 258 patients were transplanted in our center. From these, 200 receptors were male (77.5%) and constituted our study population, further divided into those who received the heart from a female donor (Group A) - 44 patients (22%) and those who received it from a male donor (Group B) - 156 (78%). Median follow-up was 4.2 ± 3.0 years (1-10 years). The two groups were quite comparable with each other, except for body mass index, systolic pulmonary artery pressure, and transpulmonary gradient, which were significantly lower in Group A. A low donor/recipient weigh ratio (<0.8) was avoided whenever possible. Hospital mortality was not different in the two groups. During follow-up, global survival was similar, as was survival free from acute cellular rejection and cardiac allograft vasculopathy. However, patients in Group A had decreased survival free from serious infections and malignant tumors. Allocation of female donors to male receptors can be done safely, at least in receptors without pulmonary hypertension and when an adequate donor/recipient weigh ratio is ensured.
- Improving risk stratification in non-ST-segment elevation myocardial infarction with combined assessment of GRACE and CRUSADE risk scoresPublication . Paiva, L; Providência, R; Barra, S; Dinis, P; Faustino, AC; Costa, M; Gonçalves, LBACKGROUND: Risk assessment is fundamental in the management of acute coronary syndromes (ACS), enabling estimation of prognosis. AIMS: To evaluate whether the combined use of GRACE and CRUSADE risk stratification schemes in patients with myocardial infarction outperforms each of the scores individually in terms of mortality and haemorrhagic risk prediction. METHODS: Observational retrospective single-centre cohort study including 566 consecutive patients admitted for non-ST-segment elevation myocardial infarction. The CRUSADE model increased GRACE discriminatory performance in predicting all-cause mortality, ascertained by Cox regression, demonstrating CRUSADE independent and additive predictive value, which was sustained throughout follow-up. The cohort was divided into four different subgroups: G1 (GRACE<141; CRUSADE<41); G2 (GRACE<141; CRUSADE≥41); G3 (GRACE≥141; CRUSADE<41); G4 (GRACE≥141; CRUSADE≥41). RESULTS: Outcomes and variables estimating clinical severity, such as admission Killip-Kimbal class and left ventricular systolic dysfunction, deteriorated progressively throughout the subgroups (G1 to G4). Survival analysis differentiated three risk strata (G1, lowest risk; G2 and G3, intermediate risk; G4, highest risk). The GRACE+CRUSADE model revealed higher prognostic performance (area under the curve [AUC] 0.76) than GRACE alone (AUC 0.70) for mortality prediction, further confirmed by the integrated discrimination improvement index. Moreover, GRACE+CRUSADE combined risk assessment seemed to be valuable in delineating bleeding risk in this setting, identifying G4 as a very high-risk subgroup (hazard ratio 3.5; P<0.001). CONCLUSIONS: Combined risk stratification with GRACE and CRUSADE scores can improve the individual discriminatory power of GRACE and CRUSADE models in the prediction of all-cause mortality and bleeding. This combined assessment is a practical approach that is potentially advantageous in treatment decision-making.
- Endometriosis-induced intussusception of the caecal appendixPublication . Costa, M; Bento, A; Batista, H; Oliveira, FAppendicular intussusception is an uncommon entity, with a reported incidence of 0.01%. The diagnosis is difficult and often only performed at the time of surgery. Intussusception has multiple causes including tumours, foreign bodies and polyps. The definitive treatment is surgical, and the extent of resection is determined by the underlying pathology and degree of invagination. Endometriosis is a rare cause of appendicular intussusception, with 194 cases described in the English literature. We report a case of a 42-year-old woman who presented with chronic abdominal pain in the lower right quadrant. A mass at the caecum was identified during investigations for renal stones by CT. Colonoscopy showed a polypoid lesion, with presumed origin in the appendix. Ileocaecal resection was performed because an appendicular tumour was suspected. Pathological examination identified endometriosis of the appendix and associated peritoneum with invagination of the caecum. The patient was discharged 7 days after surgery and is currently asymptomatic.
- Decreasing the time to achieve therapeutic vancomycin concentrations in critically ill patients: developing and testing of a dosing nomogramPublication . Baptista, JP; Roberts, JA; Sousa, E; Freitas, R; Deveza, N; Pimentel, JINTRODUCTION: Achievement of optimal vancomycin exposure is crucial to improve the management of patients with life-threatening infections caused by susceptible Gram-positive bacteria and is of particular concern in patients with augmented renal clearance (ARC). The aim of this study was to develop a dosing nomogram for the administration of vancomycin by continuous infusion for the first 24 hours of therapy based on the measured urinary creatinine clearance (8 h CLCR). METHODS: This single-center study included all critically ill patients treated with vancomycin over a 13-month period (group 1), in which we retrospectively assessed the correlation between vancomycin clearance and 8 h CLCR. This data was used to develop a formula for optimised drug dosing. The efficiency of this formula was prospectively evaluated in a second cohort of 25 consecutive critically ill patients (group 2). Vancomycin serum concentrations between 20 to 30 mg/L were considered adequate. ARC was defined as 8 h CLCR more than 130 ml/min/1.73 m(2). RESULTS: The incidence of ARC was 36% (n = 29/79) and 40% (10/25) in group 1 (n = 79) and 2 (n = 25), respectively. The mean serum vancomycin concentration on day 1 was 21.5 (6.4) and 24.5 (5.2) mg/L, for both groups respectively. On the treatment day, vancomycin plasma clearance was 5.12 (1.9) L/h in group 1 and correlated significantly with the 8 h CLCR (r(2) = 0.66; P < 0.001). The achievement of adequate vancomycin serum concentrations in group 2 was 84% (n = 21/25) versus 51% (n = 40/79) - P < 0.005. CONCLUSIONS: This new vancomycin nomogram enabled the achievement of adequate serum concentrations in 84% of the patients on the first day of treatment.
- First report of chronic hepatitis E in renal transplant recipients in PortugalPublication . Breda, F; Cochicho, J; Mesquita, JR; Bento, A; Oliveira, RP; Louro, E; Nascimento, MS; Leitão, J; Gomes, HV; Carvalho, AHepatitis E virus (HEV) infection can be responsible for chronic hepatitis in immunocompromised patients, and can rapidly evolve into fibrosis and/or hepatic cirrhosis. We present two cases of chronic hepatitis E, emphasizing the need to be aware of this entity as a growing etiology of hepatitis in transplant and immunocompromised patients.
- Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosisPublication . Gameiro, A; Gouveia, M; Tellechea, O; Moreno, APrimary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood. There are numerous unusual clinical variants of MF, including the hypopigmented type form (HMF). HMF is exceptional overall, but comparatively common among children. We present an 8-year-old boy with a 3-year history of progressive, generalised, scaly, hypopigmented round patches and few erythematous papules. He was first diagnosed with pityriasis alba (PA), and moisturisers were prescribed with no improvement. Skin biopsy showed typical features of MF, and the patient was successfully treated with narrowband ultraviolet B. HMF may simulate atopic dermatitis, PA, pityriasis lichenoides, tinea versicolour, vitiligo, postinflammatory hypopigmentation or leprosy. Therefore, persistent and unusual hypopigmented lesions should be biopsied to rule out this rare variant of MF.